제1형 신경섬유종증에 동반된 청소년기 모양세포성 성상세포종의 악성 전환 1례
A Case of Malignant Transformation of Juvenile Pilocytic Astrocytoma with Neurofibromatosis Type 1
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초록
PA는 소아에서 가장 흔한 두개내종양으로 성장 속도가 느리고 경계가 명확하며 수술적 치료로 대단히 양호한 예후를 가지는 것으로 알려져 있다. PA의 악성 전환은 대단히 드문 경우로 국내에서는 아직 보고된 바가 없다. 저자들은 NF-1이 동반된 19세 여자 환자에서 PA 진단 약 5년 뒤 교모세포종으로 악성 전환을 보인 환자를 경험하였고 문헌고찰을 통해 볼 때 PA에 대한 방사선 조사가 악성 전환의 한 원인이었을 것으로 사료된다.
Abstract
Pilocytic astrocytoma (PA) is a World Health Organization (WHO) grade I tumor typically affecting children and young adults. In general, surgical resection for PA is thought to be curative, and tumor recurrence or malignant transformation is relatively rare. Herein, we report an extremely rare case of a malignant transformation of PA. Five years prior, the patient underwent subtotal resection for PA in the left suprasellar area and left side brain parenchyma of the third ventricle, followed by gamma knife radiosurgery for remnants of the tumor. Four years after surgery, recurrent PA developed, and surgery was performed through an anterior interhemispheric transcallosal approach. One year after surgery, a rapidly growing tumor in the left basal ganglia and hypothalamus was discovered, and subtotal removal was performed. The histological diagnosis was glioblastoma. To the best of our knowledge, malignant transformation of PA has not been reported in Korea. Through review of the literature, we found malignant transformation to be limited solely to patients treated with radiotherapy, which is an observation that should be validated in future studies.
참고문헌 (16)
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